Considered rare, the synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is a distinct clinical entity, associating skin manifestations and osteoarticular symptoms. Anterior chest wall pain centered at sternoclavicular and sternocostal joints is an important and characteristic clinical finding that can lead to its diagnosis. Radiologists and clinicians must be aware of synovitis-acne-pustulosis-hyperostosis-osteitis syndrome as it can mimic some of the more common disease entities such as Paget\'s disease. We report the case of a 63-year-old male patient, with no significant medical history, who presented to the dermatology department, with severe palmar and plantar pustulosis associated with polyarthralgia. Computerized tomography scan showed sternoclavicular hyperostosis, in favor of SAPHO syndrome, with regression of clinical symptoms after non-steroidal anti-inflammatory drug treatment.

En plaque meningioma is a rare type of meningioma characterized by an infiltrative nature, sheet-like growth, and at times invading the bone. We report here a case of en plaque meningioma. The patient was a 66-year-old woman presenting with headache and painful bilateral proptosis. Cerebral magnetic resonance imaging revealed a bilateral en plaque meningioma showed as a bilateral hyperostotic of greater wing sphenoid bone associated with bilateral thickening and enhancement of the dura in the anterior temporal area and the retroclival region invading bilaterally the cavernous sinus, the prechiasmatic portion of the optic nerve, and the lateral rectus muscle of the orbit through the superior orbital fissure causing bilateral exophthalmia. Due to invasion of the cavernous sinus and the orbital apex, a subtotal but extensive removal combined with bony decompression of the cranial nerves at the superior orbital fissure and optic canal followed by adjuvant radiotherapy frequently produces good functional and cosmetic results, and over a 3-year period, follow-up magnetic resonance imaging scans showed no obvious signs of recurrence.

BACKGROUND: Osteopetrosis is a rare hereditary disease that can be transmitted in an autosomal recessive or autosomal dominant.
METHODS: Here, we report a case of trochanteric fracture in an 18-year-old boy with an anatomical plate. At the last follow-up, 24 months after surgery, the fracture had healed well, and the patient was not restricted in his activities.
CONCLUSIONS: Osteopetrosis is a rare bone disease that is mainly caused by osteoclast dysfunction. It results from a remodelling defect that leads to hypermineralization of the skeleton, resulting in bone fragility. Both surgical and nonsurgical management have advantages and disadvantages. Thus, open reduction and anatomic plate fixation remain effective management modalities for trochanteric fractures in osteopetrosis patients.
CONCLUSIONS: For our patient and as described in the literature, the complication rate decreases as some principles are respected with better consolidation of the osteoporotic fracture.

BACKGROUND: The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare condition. Its treatment remains a challenge for clinicians, and often yields mixed results.
METHODS: We report the case of a 51-year-old Caucasian woman who presented with SAPHO syndrome with mainly axial involvement. She had been treated with sulfasalazine and anti-inflammatory drugs for many years without any success. A few weeks after starting treatment with tofacitinib, both clinical and biological parameters dramatically improved. Imaging also showed considerable regression of the vertebral and pelvic lesions. However, tofacitinib had to be discontinued due to the occurrence of pulmonary embolism. Consequently, recurrence of bone pain and biologic inflammation was rapidly observed.
CONCLUSIONS: Anti-JAKs are an interesting treatment option in the management of SAPHO syndrome that need further clinical trials and assessment for validating response.

Melorheostosis is a rare benign bone pathology involving bone dysplasia and hyperostosis. The disease can be recognized with a characteristic radiographic feature of radiopaque lesions dripping along a long bone\'s diaphysis. The aberrant bone formation and development manifests mainly as pain, edema, and paresthesia of the affected limb. Severe cases may report limb deformity as well as limited range of motion. Until now, there have been approximately 300 cases reported about melorheostosis worldwide and its diverse clinical picture and age distribution. In Vietnam, there is only one known case of melorheostosis discovered incidentally via radiography. The scarcity of cases presents a challenge within the medical community in recognizing and diagnosing the condition, and a delayed diagnosis can lead to severe contracture and compromised limb motility. In this article, we reported an 82-year-old case of polyostotic melorheostosis with late onset and predominant edema, affecting the sternum, the ribs, and multiple bones of the right extremities and presented our clinical approach for a geriatric patient with chronic limb edema. Our case is distinctive in terms of anatomical location as well as the predominant 20-year non-pitting edema. A prompt diagnosis was made upon the classic dripping candle wax radiographic features emphasizing the role of plain X-ray in establishing the diagnosis without extraneous utilization of other modalities and invasive procedures. Exclusion of other causes of chronic edema such as lymphadenopathy, malignancy as well as parasitic infection is of clinical importance.

Hyperostosis of the skull is a rare bone dysplasia described in disorders such as hyperostosis cranialis interna (HCI) and hyperostosis frontalis interna (HFI). Other syndromes presenting with hyperostosis include Morgagni-Stewart-Morel (MSM) and Troell-Junet. HCI is an abnormal hyperostosis of most endosteal skull and calvarium surface regions. A more specific hyperostosis, HFI, is an unusual bone growth based on its volume and porosity; it is primarily located bilaterally on the frontal portions of the calvarium. However, the hyperossification does not cross the superior sagittal sinus. Upon cadaveric dissection, we found hyperossification beyond the frontal area, extending to the parietal and occipital bones with the significant characteristic of no midline interference. Hyperossification results in gross indentations on the corresponding frontal, parietal, and occipital hemispheric brain tissues. This report discusses possible differentials for this rare cadaveric finding of frontal, parietal, and occipital bone hyperostosis. This case report includes some major characteristic features indicative of HCI and HFI with some interesting variations and features suggestive of MSM and Troell-Junet syndromes. Due to the lack of patient history and medical records, no further conclusions about clinical differentials, symptoms, or causative syndromes could be drawn; further research needs to be conducted on HCI, HFI, and related syndromes to understand their presentations better.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare autoimmune inflammatory disease characterized by osteoarticular and dermatological manifestations. The most common osteoarticular manifestations involve the anterior chest wall, axial skeleton, and long bones. Cranial bone involvement is less reported in SAPHO syndrome. We herein present three cases of SAPHO syndrome with cranial bone involvement, and review the previous literature on similar manifestations. It was revealed that SAPHO syndrome could lead to cranial bone involvement, which could involve the dura mater, leading to hypertrophic pachymeningitis, but the outcome is usually good. Janus kinase inhibitors may be a potential treatment option.

暂无摘要。

Hyperostosis Frontalis Interna (HFI), a condition that has been sparsely explained till now, is a benign, asymptomatic, and irregular thickening of the endocranium of the frontal bone. It is found to be predominantly present in post-menopausal women during incidental X-ray or CT/MRI of the skull. The prevalence of HFI is documented in different populations, but in the Indian population, it is comparatively rare. Thus, we discuss a serendipitous finding of HFI in an Indian skull. This rare variation was noted in dry Indian human skulls. Gross features of the skull were noted, and it was an adult female skull. The area was decalcified, paraffin-embedded, and stained with Haematoxylin and Eosin. The skull bone was also subjected to plain X-ray/CT investigation. The X-ray skull of 50+ year female type features in anteroposterior and lateral view showed widening of the diploic spaces 8-10 mm with ill-defined hyperdense areas in the frontal region. Changes in computed tomography were noted. HFI often has nonspecific and benign symptoms. However, in severe cases, widespread clinical implications starting from headache, motor aphasia, parkinsonism, and depression can occur, and thus we all should be aware of it.

We report the case of a 59-year-old female patient, presenting with pustular rash on both hands and pain in the lumbosacral part and left lower limb. A magnetic resonance imaging examination of the left leg was undertaken and the result showed that a malignant lesion with bone destruction of the left femoral shaft could not be excluded. Subsequently, bone tumor was excluded by pathological examination. Lung computed tomography scan showed patchy consolidation and cord shadow in the middle left lung. Subsequently, lung cancer was excluded by pathological examination, and the histopathological changes of lung were consistent with those of organized pneumonia. Blood tests revealed elevated C-reactive protein and erythrocyte sedimentation rate. Antinuclear antibody, rheumatoid factor, and human leukocyte antigen-B27 were unremarkable. Whole body bone scintigraphy via technetium 99m-methyl diphosphonate showed increased radionuclide uptake in the left middle femur. Based on her clinical manifestations, imaging results and bone scintigraphy, the patient was diagnosed as having synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome. Loxoprofen and Tripterygium wilfordii Hook F led to impressive clinical and radiologic improvement.